title: Adductor laryngeal breathing dystonia in NBIA treated with botulinum toxin-A.
abstract: We report a rare case of neurodegeneration with brain iron accumulation (NBIA) presented with episodic inspiratory stridor. A 10-year-old boy presented with 3-year history of gradually progressive spastic gait and generalized dystonia (involving all four limbs, neck, jaw, and speech). MRI brain showed "Eye of Tiger" sign. He recently developed severe inspiratory stridor associated with almost gasping respiration. Direct video laryngoscopy showed paradoxical vocal cord closure during inspiration. He was treated with EMG-guided botulinum toxin-A injection given into bilateral thyroarytenoid muscles, resulting in dramatic response with complete disappearance of the stridor within a week. The effect lasted 18 months.
authors.affiliation: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Vinod
authors.initials: V
authors.lastname: Rai
authors.forename: Vinay
authors.initials: V
authors.lastname: Goyal
authors.forename: Garima
authors.initials: G
authors.lastname: Shukla
authors.forename: Girija
authors.initials: G
authors.lastname: Rath
authors.forename: Madhuri
authors.initials: M
authors.lastname: Behari
keywords: Adductor laryngeal breathing dystonia; botulinum toxin; stridor